Syndrome consisting of alopecia or hypotrichosis,
oligophrenia, microcephaly, seizures, and mental retardation.
Alopecia-Epilepsy-Oligophrenia Syndrome; Alopecia-Mental
Retardation-Epilepsy-Microcephaly Syndrome; Epilepsy-Oligophrenia Syndrome
Three types of this disorder are suggested:
Type I: Total alopecia and mental retardation, the main features, in association
Type II: Subtotal alopecia and mental retardation with or without epilepsy
Type III: Subtotal alopecia and psychomotor retardation with microcephaly and
First described in 1962 by E.J. Moynahan, an British dermatologist practicing
The incidence remains unknown.
Familial with parental consanguinity;
autosomal recessive trait.
Microcephaly, alopecia or hypertrichosis, and epilepsy
(grand mal seizures).
Sparse or absent scalp hair, microcephaly,
seizures, short stature; hypogonadism with late puberty. Poor musculature.
Sometimes sensorineural deafness.
No literature on anesthesia in this
condition. However, full neurologic history and examination, epileptic
history, especially of control and medications, and associated anomalies
resulting from microcephaly must be reviewed.
Epileptic control is essential; usual
medications should be maintained until the morning of surgery wherever
possible and appropriate nontriggering anesthetic agents used. Psychomotor
retardation with poor speech skills may lead to a uncooperative patient.
Note potential for interaction with
antiepileptic drugs. Chronic phenytoin administration increases
nondepolarizing neuromuscular blocker requirements, produces gingival
hyperplasia and bleeding, and may cause hepatic dysfunction. Ketamine,
enflurane, and methohexital are relatively contraindicated. High
concentration (greater than 5-6%) of sevoflurane at induction might trigger epilepsy.
Baraitser M, Carter CO, Brett EM: A new alopecia/mental retardation
syndrome. J Med Genet
Moynahan EJ: Familial congenital alopecia, epilepsy, mental retardation with
unusual electroencephalogram. Proc R Soc Med
Perniola T, Krajewska G, Carnevale F, et al: Congenital alopecia,
psychomotor retardation, convulsions in two sibs of a consanguineous
marriage. J Inherit Metab Dis